Eur J Pediatr Surg 2007; 17(6): 393-396
DOI: 10.1055/s-2007-965811
Original Article

© Georg Thieme Verlag KG Stuttgart · New York

Bilateral Adrenal Neuroblastoma is Different

F. Pederiva1 , A. Andres1 , A. Sastre1 , J. Alves2 , L. Martinez1 , J. A. Tovar1
  • 1Department of Pediatric Surgery, Hospital Universitario “La Paz”, Madrid, Spain
  • 2Department of Pathology, Hospital Universitario “La Paz”, Madrid, Spain
Further Information

Publication History

received May 14, 2007

accepted May 23, 2007

Publication Date:
11 December 2007 (online)

Abstract

Background/Aims: Bilateral adrenal neuroblastoma is rare and can be due to multifocal primary or contralateral metastasis. Staging is confusing in these patients and treatment guidelines are difficult to set. The present study examines the clinical, biological and therapeutic features of bilateral adrenal neuroblastoma. Methods: We identified 4 cases primarily located in both adrenals out of 148 neuroblastomas treated between 1992 and 2006. We studied the clinicopathological findings and biological features, including MYCN amplification, and analyzed the treatment strategies and results. Results: All patients were younger than 6 months of age and all had multiple liver metastases. Three had subcutaneous nodules and massive liver enlargement. All underwent chemotherapy prior to operation. Two babies had large bilateral tumors without preservable glands and underwent bilateral adrenalectomy. Both had MYCN gene amplification and died of widespread (brain and bone) metastases some weeks later. In the remaining two patients adrenalectomy was performed on the side of the larger tumor with tumor enucleation on the other side to preserve hormonal function followed by 2 courses of mild chemotherapy in one patient. These tumors were not amplified. Both of these children are doing well. Conclusions: Bilateral adrenal neuroblastomas fit neither into stage 4 s nor into stage 4. Their clinical behavior is exceptional with a number of multicystic forms, variable MYCN amplification, widespread metastases and a high mortality. Bilateral adrenalectomy is sometimes unavoidable, but unilateral removal with contralateral enucleation, partial resection or observation are valid alternatives. Mortality is higher than in regular stage 4 s cases. This particular group of neuroblastomas required individually tailored therapeutic strategies based on the size, extent and prognostic markers.

References

Prof. Dr. Juan A. Tovar

Department of Pediatric Surgery
Hospital Universitario “La Paz”

Paseo de la Castellana, 261

28046 Madrid

Spain

Email: [email protected]